Cholesteatoma Practice Essentials A cholesteatoma consists of squamous epithelium that is trapped within the skull base and that

can erode and destroy important structures within the temporal bone. Its potential for causing central nervous system (CNS complications (eg! brain abscess! meningitis makes it a potentially fatal lesion. "enerally! the following # types of cholesteatoma are identified$ Congenital cholesteatoma %rimary acquired cholesteatoma Secondary acquired cholesteatoma Signs and symptoms &he hallmark symptom of a cholesteatoma is a painless otorrhea! either unremitting or frequently recurrent. 'ther symptoms include the following$ Conductive hearing loss (i))iness$ *elatively uncommon (rainage and granulation tissue in the ear canal and middle ear$ +nresponsive to antimicrobial therapy 'ccasionally! cholesteatoma initially presents with symptoms of CNS complications! including the following$ Sigmoid sinus thrombosis ,pidural abscess -eningitis +nlike other cholesteatomas! the congenital type is usually identified behind an intact! normal.appearing tympanic membrane. &he child often has no history of recurrent suppurative ear disease! previous otologic surgery! or tympanic membrane perforation./0! 1! #! 23 See Clinical %resentation for more detail. Diagnosis No laboratory tests or incisional biopsies are generally necessary for the diagnosis of cholesteatomas! because the diagnosis can be made based on physical e4amination and radiologic findings. Computed tomography (C& scanning is the diagnostic imaging modality of choice for these lesions! owing to its ability to detect subtle bony defects. 5istologically! surgically removed cholesteatoma specimens demonstrate typical squamous epithelium. &he histology is indistinguishable from that of sebaceous cysts or keratomas removed from any other portion of the body. Audiometry should be performed prior to surgery whenever possible. Air and bone conduction! the speech reception threshold! and speech discrimination scores should all be determined within a few weeks of the proposed operative procedure. -agnetic resonance imaging (-*I is used when very specific problems! such as the following! are suspected /63 $ (ural involvement or invasion Subdural or epidural abscess 7rain herniation into the mastoid cavity Inflammation of the membranous labyrinth or facial nerve Sigmoid sinus thrombosis -eningitis See 8orkup for more detail. Management

9irtually all cholesteatomas should be e4cised. &he only absolute contraindications to the surgical removal of cholesteatomas are medical in nature. Canal wall.down tympanomastoidectomy In the canal wall:down (open procedure! the posterior canal wall is removed. A large meatoplasty is created to allow adequate air circulation into the cavity that arises from the operation. Canal wall:down operations have the highest probability of permanently ridding patients of cholesteatomas. Canal wall.up tympanomastoidectomy In the canal wall:up (closed procedure! the canal wall is preserved. Canal wall:up procedures have the advantage of maintaining a normal appearance! but the risk of persistent or recurrent cholesteatomas is higher than in the canal wall:down operation. See &reatment for more detail. Image library

,pitympanic (attic cholesteatoma. &his is a typical primary acquired cholesteatoma in its earliest stages. Background A cholesteatoma consists of squamous epithelium that is trapped within the skull base that can erode and destroy important structures within the temporal bone. Its potential for causing central nervous system (CNS complications (eg! brain abscess! meningitis makes it a potentially fatal lesion (see the images below . (See ,tiology and %athophysiology and %rognosis.

cholesteatoma. cholesteatoma

&his is in

,pitympanic a typical primary its earliest

(attic acquired stages.

congenital

cholesteatoma. A white mass can be seen behind an intact drum. &hroughout the early half of the 1;th century! cholesteatomas were managed by e4teriori)ation. &he mastoid air cells were e4enterated! the posterior wall of the e4ternal auditory canal was removed! and the opening into the resulting cavity was enlarged to ensure adequate air e4change and to make visual inspection simple. (uring the 0<6;s and 0<=;s! the 5ouse 'tologic "roup developed a new approach. &he group attempted to leave the basic underlying anatomic structure of the ear and temporal bone intact! principally by preserving the canal wall. &hese aggressive attempts to conserve the normal anatomy of the ear created great controversy. Surgeons tended to align themselves with either the old >canal wall: down> (ie! open cavity procedure/=3 or with the new >canal wall:up> (ie! closed cavity method./?3 -ost otologic surgeons have now migrated to an intermediate position! with the ma@ority of those in the +nited States now performing both techniques and basing the selection of the open or closed cavity procedure on the individual circumstances of each patient. (See 8orkup and &reatment. Etiology and Pathophysiology A cholesteatoma consists of squamous epithelium that is trapped in the skull base. Squamous epithelium trapped within the temporal bone (ie! middle ear or mastoid can e4pand only at the e4pense of the bone that surrounds and contains it. Consequently! a growing cholesteatoma can erode and destroy important structures within the temporal bone. Cholesteatomas cause bony erosion by either of the following mechanisms$ %ressure effects produce bony remodeling! as occurs normally throughout the entire skeleton when pressure is applied consistently over time ,n)ymatic activity at the margin of the cholesteatoma enhances osteoclastic activity! which greatly increases the speed of bone erosionA osteolytic en)ymes appear to increase when a cholesteatoma becomes infected 'ccasionally! a cholesteatoma escapes the confines of the temporal bone and skull base. ,4tratemporal complications can occur in the neck! CNS! or both. 8hen a cholesteatoma within the cranium grows large enough to produce mass effect! brain dysfunction can develop. "enerally! the following # types of cholesteatoma are identified$ congenital! primary acquired! and secondary acquired. Congenital cholesteatoma Congenital cholesteatomas arise as a consequence of squamous epithelium trapped within the temporal bone during embryogenesis. &he typical congenital cholesteatoma is found in the anterior mesotympanum or in the perieustachian tube area. &hey are identified most commonly in early childhood (= mo to 6 y . /B3 As they e4pand! congenital cholesteatomas can obstruct the ,ustachian tube and produce chronic middle ear fluid and conductive hearing loss. &hey can also e4pand posteriorly to encase the ossicular chain and! by this mechanism! produce conductive hearing loss.

+nlike other cholesteatomas! the congenital type is usually identified behind an intact! normal.appearing tympanic membrane. &he child often has no history of recurrent suppurative ear disease! previous otologic surgery! or tympanic membrane perforation./0! 1! #! 23 Primary acquired cholesteatoma A primary acquired cholesteatoma results from tympanic membrane retraction. &he classic case develops from progressively deeper medial retraction of the pars flaccida into the epitympanum (attic . As this process continues! the lateral wall of the epitympanum (the scutum is slowly eroded! producing a defect in the lateral wall of the epitympanum that slowly e4pands. &he tympanic membrane continues retracting medially until it passes over the heads of the ossicles and into the posterior epitympanum. 'ssicular destruction is common. If the cholesteatoma pokes posteriorly into the aditus ad antrum and the mastoid itself! erosion of the tegmen mastoideum! with e4posure of the dura andCor erosion of the lateral semicircular canal with deafness and vertigo! may result./<3 A second type of primary acquired cholesteatoma arises when the posterior quadrant of the tympanic membrane is retracted into the posterior middle ear. &he drum initially adheres to the long process of the incus. As retraction continues medially and posteriorly! squamous epithelium envelops the superstructure of the stapes and retracts into the sinus tympani. %rimary cholesteatomas arising from the posterior tympanic membrane are likely to produce facial nerve e4posure (and occasionally paralysis and destruction of the stapedial superstructure. Surgical removal from the sinus tympani may be e4tremely challenging. Secondary acquired cholesteatoma Secondary acquired cholesteatomas result directly from an in@ury to the tympanic membrane. &his in@ury can be a perforation caused by acute otitis media or trauma! or it may be due to surgical manipulation of the drum. A procedure as simple as the insertion of tympanostomy tubes could implant squamous epithelium into the middle ear! ultimately producing a cholesteatoma. %osterior marginal perforations are the most likely to result in cholesteatoma formation. Although central perforations are considered unlikely to produce cholesteatomas! they do so occasionally. Any deep retraction pocket can lead to cholesteatoma formation if the retraction pocket becomes deep enough to trap desquamated epithelium. /0;! 003 Epidemiology &he incidence of cholesteatomas in the +nited States is unknown! but they are a relatively common reason for otologic surgery (appro4imately weekly in tertiary otologic practices . (eath from intracranial complications of cholesteatoma is now uncommon! with the change being attributable to earlier recognition! timely surgical intervention! and supportive antibiotic therapy. Cholesteatomas remain a relatively common cause of permanent! moderate conductive hearing loss in children and adults. Prognosis ,liminating a cholesteatoma is almost always possibleA however! multiple operations may be required. 7ecause surgery is generally successful! complications from

uncontrolled cholesteatoma growth are now relatively uncommon. Canal wall:down (open cavity tympanomastoidectomy offers a very low rate of recurrence or persistence of cholesteatomas. *eoperation for cholesteatomas occurs in 6D of patients or less. &his compares quite favorably to the 1;.2;D recurrence rates associated with closed.cavity techniques (canal wall : up ./=3 Nonetheless! because the ossicular chain andCor tympanic membrane cannot always be completely restored to normal! cholesteatomas remain a relatively common cause of permanent! moderate conductive hearing loss. Mortality (eath from intracranial complications of cholesteatoma is now uncommon! with the change being attributable to earlier recognition! timely surgical intervention! and supportive antibiotic therapy. History Painless otorrhea &he hallmark symptom of a cholesteatoma is a painless otorrhea! either unremitting or frequently recurrent. 8hen the cholesteatoma becomes infected! the infection may be e4tremely difficult to eradicate. 7ecause the cholesteatoma has no blood supply! systemic antibiotics cannot be delivered to the center of the cholesteatoma. &opical antibiotics often surround a cholesteatoma! suppress infection! and penetrate a few millimeters toward its centerA however! a large! infected cholesteatoma is resistant to any type of antimicrobial therapy. Consequently! otorrhea either persists or recurs! despite frequent and aggressive treatment with antibiotics. Hearing loss 5earing loss is also a common symptom of cholesteatomas. Earge cholesteatomas fill the middle ear space with desquamated epithelium! with or without associated mucopurulent discharge! interfering with sound transmission. 'ssicular damage is frequently present and can also produce or may magnify a conductive loss. (See the image below.

'ccasionally! cholesteatoma initially presents with symptoms of CNS complications! sigmoid sinus thrombosis! /013 epidural abscess! or meningitis. Physical E!amination +pon physical e4amination! the most common sign of a cholesteatoma is drainage and granulation tissue in the ear canal and middle ear that are unresponsive to antimicrobial therapy. A tympanic membrane perforation is present in more than <;D of cases. Congenital cholesteatomas are an e4ception! with the drum often remaining intact until the middle ear component is quite large. 'ccasionally! a cholesteatoma that is produced by surgical implantation of squamous epithelium manifests prior to disruption of the tympanic membrane! but even in such cases! the cholesteatoma eventually produces a tympanic membrane perforation./0#3 Fuite frequently! the only finding on physical e4amination is a canal filled with mucopus and granulation tissue. Sometimes! eliminating the infection and resolving the granulation tissue with either systemic antibiotics or ototopical antibiotic drops is impossible. 8hen ototopical therapy is successful! a deep tympanic retraction pocket may be seen in the pars flaccida (the portion of the tympanic membrane that is superior to the short process of the malleus or in the posterior quadrant of the tympanic membrane. 9ery seldom is a cholesteatoma first identified based on one of its complicationsA however! this occasionally happens in children. An infection associated with the cholesteatoma can erupt through the inferior mastoid corte4 and manifest as an abscess in the neck. 'ccasionally! cholesteatomas first manifest with the signs and symptoms of CNS complications! sigmoid sinus thrombosis! epidural abscess! or meningitis. Diagnostic Considerations &he diagnosis may be in doubt in individuals with only small epitympanic retractions observed during the physical e4amination. Computed tomography (C& scanning may help to distinguish between shallow retractions without soft tissue e4tension into the epitympanic space and an e4tensive soft tissue mass with bony erosion. C& scanning can be helpful in individuals who have already had tympanomastoid surgery. In this group of patients! a white mass behind the tympanic membrane (shown below may represent tympanosclerosis! cartilage! or a recurrent cholesteatoma. C& scan findings frequently help to resolve such questions. (iffusion.weighted magnetic resonance imaging (-*I may also be helpful in such circumstances.

A large epitympanic (attic cholesteatoma that is much more advanced than the lesion in the previous image. Dizziness (i))iness is a relatively uncommon symptom of cholesteatomas! but it does occur if bony erosion produces a labyrinthine fistula or if the cholesteatoma is lying directly on the footplate of the stapes. (i))iness is a worrisome symptom because it may presage the development of more serious complications. ther

A large cholesteatoma. No landmarks are visible! which typically is the case with advanced cholesteatomas.

-iddle ear osteoma and chronic suppurative otitis media are other differentials in the diagnosis of cholesteatoma. "pproach Considerations No laboratory tests or incisional biopsies are generally necessary for the diagnosis of cholesteatomas! because the diagnosis can be made based on physical e4amination and radiologic findings. C& scanning is the diagnostic imaging modality of choice for these lesions! owing to its ability to detect subtle bony defects. 5istologically! surgically removed cholesteatoma specimens demonstrate typical squamous epithelium. &he histology is indistinguishable from that of sebaceous cysts or keratomas removed from any other portion of the body. "udiometry Audiometry should be performed prior to surgery whenever possible. Air and bone conduction! the speech reception threshold! and speech discrimination scores should all be determined within a few weeks of the proposed operative procedure. Infrequently! a serious complication of cholesteatoma requires urgent surgeryA in these patients! audiometric testing can be omitted. C# Scanning As previously mentioned! C& scanning is the imaging modality of choice in the diagnosis of cholesteatomas because it can detect subtle bony defects (see the image below . 5owever! C& scanning cannot always distinguish between granulation tissue and a cholesteatoma. ,ven technically e4cellent fine.cut C& scans cannot reliably determine the full e4tent of disease.

An unenhanced computed tomography (C& scan demonstrating that the posterior canal wall has been eroded and the e4ternal auditory canal has filled with cholesteatoma! pus! and debris. Surprisingly! the middle ear appears relatively free of disease! a characteristic of primary acquired cholesteatomas. 7ecause the surgeon cannot always predict what is needed in the operating room based on findings from the preoperative evaluation! the surgeon and the patient must be prepared for intraoperative surprises./02! 06! 63 &he subtle bony defects that can be detected on C& scans include the following/63 $ Scutal erosion/0=3 Eabyrinthine fistula (efects in the tegmen (etails of ossicular involvement (etails of ossicular erosion or discontinuity Anomalies! erosion! or invasion of the fallopian canal (also known as the facial canal

In a study of =2 patients with middle ear cholesteatomas! "aurano and Gohar@y found that the diagnosis of these cholesteatomas was suggested by specific characteristics found on C& scans. &hese included e4pansion of the aditus and mastoid antrum (<1D of patients and ossicle erosion (also <1D of patients . %reoperative C& scans in the study had <?D correlation with surgical and histopathologic findings./063 Some surgeons believe any patient with a cholesteatoma who is scheduled for surgical intervention should have a preoperative C& scan. &hey believe that useful information is obtained in every circumstance and! consequently! that the e4pense of the scan and the radiation e4posure involved are always @ustified. 'ther surgeons reserve preoperative imaging for only special cases and are quite comfortable performing surgery without it. &hese surgeons may nonetheless request a preoperative C& scan in the following circumstances$ &he diagnosis is in doubt &he patient is adamant about avoiding an operation &he scan helps the patient or his or her parents assess the risks of ossicular involvement &he underlying anatomy is indeterminate and the e4tent of disease is unclear as a result of previous surgery &here is reasonable e4pectation of congenital anomalies (eg! atresia cases! craniofacial anomalies Complications are suspected. A labyrinthine fistula or erosion of the fallopian canal is suspected "$oidance o% surgery %atients who are poor surgical candidates or who wish to avoid an operation for other reasons can be better advised about the risks of nonsurgical management if a C& scan has been performed and evaluated. &he presence of e4tensive disease! a labyrinthine fistula! encroachment on the fallopian canal! and involvement of the oval window niche increase the risk associated with nonsurgical management. &isk assessment 5elping a patient or parents assess some of the risks of ossicular involvement is often useful. If ossicular involvement is apparent on preoperative C& scans! patients should be counseled that ossicular removal may be necessary and that significant postoperative conductive hearing loss should be e4pected. M&I -*I is used when very specific problems! such as the following! are e4pected to involve surrounding soft tissues/63 $ (ural involvement or invasion Subdural or epidural abscess 7rain herniation into the mastoid cavity Inflammation of the membranous labyrinth or facial nerve Sigmoid sinus thrombosis -eningitis A number of articles suggest that diffusion.weighted -*I may be able to distinguish between recurrent or persistent middle ear cholesteatoma and scar tissue or granulation tissue. If future studies verify a high sensitivity and specificity for this modality! it may become possible to

replace routine second.look procedures with high quality diffusion.weighted -*I scans./0?! 0B3 "pproach Considerations 9irtually all cholesteatomas should be e4cised. &he only absolute contraindications to the surgical removal of cholesteatomas are medical in nature. Some individuals have health problems of such gravity as to make the risk of surgical intervention unacceptably high. Absence of hearing in the contralateral ear is a relative contraindication to surgery. Since! however! the cholesteatoma frequently presents a greater risk to residual hearing than surgery does! surgical removal usually remains the management option of choice in these situations. (epending on the procedure! appro4imately 6.2;D of cholesteatoma operations are unsuccessful! with cholesteatoma persistence or recurrence manifesting at some point in the postoperative period. %ersistence may appear as early as 6.= months postoperatively! or it may be delayed for many years. Consequently! close follow.up care is important. &he applicability of using small otoendoscopes as part of the surgical management of cholesteatomas is being e4plored. &o date! they have been used only ad@unctively! but their role may e4pand in the future. 'onsurgical care -edical therapy is not a viable treatment for cholesteatomas. %atients who refuse surgery or whose medical condition makes a general anesthetic too ha)ardous should have their ears cleaned regularly. *egular cleaning can help to control infection and may slow growth of the cholesteatoma! although it does not stop further e4pansion or eliminate risk. &he mainstay of microbial therapy should be topical! but systemic therapy is occasionally a helpful ad@unct. Consultations Consultation with an otolaryngologist is mandatory. *arely! advice from a neurosurgeon is required to help manage an intracranial consultation. Mastoidectomy Surgical therapy consists of removing the cholesteatoma. In certain circumstances! the surgeon can make the decision to use a canal wall:up (closed or canal wall:down (open technique preoperatively. Canal wall:down operations have the highest probability of permanently ridding the patient of a cholesteatoma. Canal wall:up procedures have the advantage of maintaining a normal appearance! but because the risk of persistent or recurrent cholesteatomas is higher with this operation! most surgeons advise an obligatory second.look tympanomastoidectomy = months to 0 year after the initial procedure./0<! 1;3 At the time of the second look! small amounts of residual disease can frequently be removed prior to the development of either complications or massive recurrence. (Increasing evidence suggests that diffusion.weighted -*I may be able to accurately detect cholesteatoma recurrence and! in some cases! replace a second.look procedure./103 If the patient has had several episodes of recurrence of the cholesteatoma and wishes to avoid future operations! the canal wall:down technique is most applicable. In

addition! it is safer for patients who are unwilling or unable to return for a second.look procedure./=! ?! 113 Some congenital anomalies are known to be associated with a lifelong history of eustachian tube dysfunction! and in some individuals! previous surgical procedures have irreversibly in@ured the eustachian tube. &hese again are patients in whom an open.cavity operation often is best. 'sborn et al reported the results from 21; children who underwent ?;; operations and concluded that most children with cholesteatoma can be managed with an intact: canal wall technique./1#3 Sometimes! the decision as to which procedure to use is not possible until the operation has begun and a clear understanding of the e4tent of the disease has been obtained. No operation can be successful unless the goals of the procedure are kept clearly in mind. &hese goals include the following$ &o make the ear safe by eliminating the cholesteatoma and chronic infection &o make the ear problem.free for all the usual activities of daily living! including swimming &o conserve residual hearing &o improve hearing when possible &o provide an acceptable cosmetic appearance Preoperati$e details A careful discussion of the advantages and disadvantages of canal wall:up and canal wall:down (open procedures is useful. If a canal wall:down procedure is considered preoperatively! showing the patient a picture of a well. crafted meatoplasty! as shown below! can help the patient form a clear understanding of the e4pected postoperative appearance. Advantages and disadvantages of the canal wall:up procedure include the following$ Normal appearance 5earing aids easy to fit No routine cleaning required 5igh tolerance for water e4posure +sually a staged procedure *elatively high rate of recurrent or persistent cholesteatomas Advantages and disadvantages of the canal wall:down procedure include the following$ ,nlarged meatus 5earing aids difficult to fit Annual or semiannual canal cleaning needed 'ccasional problem with water e4posure$ (i))iness may develop after e4posure to water or cold air. +sually a single procedure Eow rate of persistent or recurrent cholesteatomas Intraoperati$e details 'ften! the best plan is to reserve the decision about how to manage the canal wall until the operation has begun and a better understanding of the e4tent of disease has emerged. Some intraoperative findings that favor a canal wall:down! or open! technique! include the following /=3 $ Involvement of the sinus tympani Involvement of the medial end of the canal wall! with the cholesteatoma wedged laterally between

the heads of the ossicles in the epitympanum and medial canal wall 'stitis or irremovable cholesteatoma in the area around the opening to the eustachian tube or in the most inferior portions of the middle ear space (often calls for a true radical mastoidectomy Earge defects in the canal wall$ Hrequently! the best plan is to convert the operation to an open. cavity procedure (in contrast! small defects in the canal wall are readily repaired Eabyrinthine fistula$ If the cholesteatoma matri4 is left over the fistula so as not to e4pose it! then the canal must be removedA otherwise! epithelium is trapped within the mastoid cavity! resulting in cholesteatoma recurrenceA if the cholesteatoma matri4 is removed from the fistula! some surgeons are comfortable leaving the canal wall up -ost surgical procedures for cholesteatomas in the +nited States are now performed through an incision behind the ear combined with an incision in the e4ternal auditory canal! but the procedure can be performed through an e4tended incision starting in the ear canal. *egardless of which technique is used! all of the mastoidIs air cells should be removed. If ossicles are involved! they must be removed to avoid recurrence./103 *econstruct the ossicular chain either at the end of the primary procedure or as part of a secondary operation. &he tympanic membrane is usually reconstructed. If a canal wall:up technique is used! missing bone is replaced with a cartilage graft. If a canal wall:down procedure is selected! a large meatoplasty is created to allow adequate air circulation into the cavity that arises as a consequence of removing the posterior canal wall. (See the image below.

&he photo e4hibits a large meatoplasty performed as part of an open cavity (canal wall:down mastoidectomy. A similar meatoplasty usually is necessary if a clean! dry! problem.free cavity is to be

maintained. A typical audiogram demonstrating bilateral conductive hearing loss!

which may be observed in an individual with a cholesteatoma. ,ssential features of a canal wall:up procedure include the following/?3 $ *emoval of all infected air cells A functioning eustachian tube A well.maintained middle ear space Adequate communication between the mastoid and the middle ear space through the aditus ad antrum or a generous facial recess ,limination of any bony epitympanic defect with a cartilage or bone graft ,ssential features of a canal wall:down procedure include the following$ *emoval of all air cells! including those within the retrofacial! retrolabyrinthine! and subarcuate air cell tracts *emoval of the lateral and posterior walls of the epitympanum so that the tegmen mastoideum and tegmen tympani become a smooth! featureless plane Amputation of the mastoid tip (usually advisable Sauceri)ation of the lateral margins of the cavity Eowering of the posterior bony e4ternal auditory wall to the level of the facial nerve ,4teriori)ation of the anterior epitympanic recess by removing the cog ,nlargement of the meatus (to at least twice its normal si)e by removing conchal cartilage Eowering of the medial end of the e4ternal auditory canal toward the floor of the hypotympanum Some surgeons prefer a subcortical technique that starts by removing bone medially through the ear canal. &he epitympanum is progressively enlarged! and the posterior canal wall is removed from the inside out. 7one removal is limited to that necessary to remove all of the cholesteatoma. Such a subcortical! or inside.out! mastoidectomy combines some of the advantages of canal wall:up and canal wall:down techniques but commits the surgeon to a canal wall:down operation if the cholesteatoma has e4tensive mastoid involvement./12! 163 Canal (all)reconstruction tympanomastoidectomy In a retrospective case review of 1?# patients (mean age #6 with chronic otitis media with cholesteatoma! 8alker et al found that an alternative surgical procedure! canal wall:reconstruction (C8* tympanomastoidectomy with mastoid obliteration! produced good long.term results. &he investigators! who e4amined the surgical outcomes on 1B6 ears (average follow.up 2.1< years ! determined the rate of failed proceduresJthose requiring conversion to open cavity or subtotal petrosectomyJto be only 1.=D./1=3 &he investigators concluded that use of C8* tympanomastoidectomy avoids the long.term drawbacks of canal wall:down mastoidectomy while offering surgeons e4cellent e4posure of the middle ear and mastoid. Management o% the complications o% cholesteatoma Eabyrinthine fistula Sometimes! the e4istence of a labyrinthine fistula is suggested on preoperative C& scansA at other times! the presence of a fistula is a complete surprise. Initially! the

desquamated epithelium should be removed! carefully leaving the matri4 over the hori)ontal canal. 7lunt probes are used to locate the fistula by palpation. If the fistula appears to be superficial! the matri4 is gently removed at the end of the case and the defect is covered with fascia. 5owever! if the fistula is large and it appears that the cholesteatoma matri4 is attached firmly to the membranous labyrinth itself! leaving the matri4 in position should be considered. (ebate continues as to whether hearing loss is more likely if the matri4 is removed. If the membranous labyrinth is opened at any point during the procedure! broad.spectrum I9 antibiotics and steroids should both be administered immediately. 7acterial labyrinthitis and inflammation pose the biggest risk to hearing./1?3 Histulas involving the oval window area should be treated according to the same principles. 9iolation of the vestibule itself is much more likely to produce hearing loss than violation of one of the semicircular canals. 'ccasionally! a canal fistula is created during the surgical procedure. If such a fistula involves one of the semicircular canals! then it should be sealed with soft tissue (preferably fascia and I9 antibiotics and steroids administered. &hese patients are quite likely to have significant balance disturbance in the immediate postoperative period! but a significant number recover with little or no hearing loss if appropriate antibiotic therapy and steroids are provided in the postoperative period. 7rain herniation 7rain herniation through the tegmen of the middle fossa has a characteristic glistening! shiny appearance. &he presence of clear fluid associated with such a lesion should raise suspicion of a brain hernia and cerebrospinal fluid leak. -*I and C& scanning can often help to clarify the diagnosis preoperatively. 'ptions for managing a brain hernia fall into 1 basic categories$ reconstruction of the mastoid cavity and obliteration of the mastoid cavity. If reconstruction is chosen! gliali)ed brain can be removed simply by sharply e4cising itA any viable brain can be pushed back up into the middle cranial fossa. If the defect is large! intradural and e4tradural repair of the dural defect should be considered. &he alternative involves obliterating the middle ear! e4ternal auditory canal! and mastoid by removing the posterior canal wall! obliterating the eustachian tube! filling the defect with abdominal fat! and over sewing the e4ternal canal. &his is an especially appealing option if hearing is very poor in the afflicted ear. If all of the squamous epithelium has been removed! obliteration ensures a safe! well.healed! problem.free ear! although ma4imal conductive hearing loss is unavoidable. Postoperati$e details *egular office visits are necessary following surgery. Some surgeons prefer to see the patient on the following day and remove the dressing themselves. 'thers prefer to wait ?. 0; daysA these surgeons allow the patient to remove the dressing at home after 01.2B hours. If nonabsorbable sutures or staples are used! they must be removed at ?.0; days. If suture or staple removal is unnecessary! some surgeons delay the first postoperative visit for as long as 1.# weeks. 'pen cavities heal slowly! and 01.12 weeks are often required for full epitheliali)ation.

%ostoperative management %ostoperative treatment regimens for canal wall:down procedures vary considerably. &he wick in the e4ternal auditory canal (,AC is removed at the first postoperative visit! ?.0; days after surgery. Antibiotic otic drops are prescribed twice daily for 0; days. &he author prefers to fill cavities from the canal wall:down surgery with antibiotic ointment at the first postoperative visit. 'pen cavities require variable amounts of cleaning over the first 2.B postoperative weeks. &he first cavity cleaning occurs at the postoperative visit 1.# weeks after the procedure! and such cleaning can be accomplished regularly in an office setting every 1.# weeks! depending on how quickly the cavity epitheliali)es. 'ften! the patient is asked to fill the cavity with pero4ide daily! starting 0.1 weeks postoperatively! or to irrigate the cavity with an alcohol.vinegar solution. &his limits the amount of superficial infection. Control of granulation tissue Hrequently! granulation tissue forms in the open cavity as it heals. "ranulation tissue may be helpful because it can contract the si)e of the cavity! resulting in a much smaller postoperative cavity than would be the case if the initial defect epitheliali)ed. 'n the other hand! granulation tissue can obliterate medial portions of the cavity! including the newly grafted drum! and compromise the hearing outcome. ,4uberant granulation tissues can significantly delay epitheliali)ation. Steroid creams! steroid.containing drops! and regular applications of gentian violet can be used to help control the development and e4tent of granulation tissue. Silver nitrate cautery effectively eliminates or reduces granulation tissue. 5owever! silver nitrate produces a chemical burn! the depth of which is difficult to control! so e4tra caution must be used around delicate or sensitive structures such as the hori)ontal semicircular and facial nerve in order to avoid in@ury. Infection control 'ften! a superficial infection develops that can be effectively controlled using topical antibiotic drops. &he causative organisms are predominantly gram negative! with %seudomonas species as the single most common pathogens! but gram.positive organisms! including Streptococcus and Staphylococcus species! account for 1;.#;D of recovered organisms. Aminoglycoside and fluoroquinolone topical preparations provide appropriate antibacterial spectra for these organisms. &he complete absence of potential ototo4icity among the fluoroquinolones makes them appealing. If a nonsteroidal otic preparation is desired! either ciproflo4acin or oflo4acin solution is an e4cellent choice. If granulation tissue is present! steroid.containing drops may be preferred. In such cases! ciproflo4acinCde4amethasone (Ciprode4 is an e4cellent choice. Antibacterial creams containing fluoroquinolones can also be used. 'ccasionally! if granulation tissue appears to be overwhelming! steroid creams alone are useful. If a superficial fungal infection or candidiasis develops! nystatin.triamcinolone (-ycolog or clotrima)ole (-ycele4 cream is usually sufficient to eradicate it. Postoperati$e Complications

Postoperati$e stenosis %ostoperative stenosis is usually a consequence of e4uberant granulation tissue and is more likely to occur when a postoperative infection is present. %rinciples of prevention include control of the infection and suppression of the granulation tissue. Surprisingly! the gentle pressure of e4panded packing often slowly increases the si)e of the canal. 7y keeping the packing in place for several months! a 0. to 1.mm nonfunctional medial canal can be e4panded to 2.6 mm! which is large enough to conduct sound efficiently and prevent additional conductive hearing loss. If postoperative stenosis completely obliterates the cavity! revision of the cavity in an attempt to restore patency and improve hearing may be necessary. Split.thickness skin grafts limit the recurrence of postoperative stenosis. *acial ner$e damage &he most dreaded complication of tympanomastoid surgery is in@ury to the facial nerve. &he incidence of permanent facial nerve in@ury following cholesteatoma surgery is not entirely certain! but it appears to be 0D or less! and it probably is much less than 0D in the hands of e4perienced otologists who perform the operation regularly. Controversy e4ists as to whether or not facial nerve monitoring is helpful in reducing the risk of postoperative facial nerve in@ury. 7ecause even the suggestion of intraoperative facial nerve in@ury may produce in the surgeon in pronounced sympathetic discharge with high levels of circulating catecholamines! technical skills may be degraded and @udgment impaired. &herefore! the surgeon should stop operating to assess the situation! and! if at all possible! a consultant opinion should be obtained immediately. ,valuation &he first step in managing a possible or recogni)ed facial nerve in@ury is to decompress the nerve around the area thought to be in@ured. 7one should be removed a few millimeters pro4imal and distal to the damaged segment so that the in@ury can be clearly visuali)ed. ,valuation of the severity of in@ury is e4tremely difficult if the in@ured segment is in the middle ear! where the space in which the surgeon can operate is very limited. &he hori)ontal semicircular canal limits e4posure posteriorly! and the superstructure of the stapes limits e4posure anteriorly. Consequently! accurate assessment of the severity of in@ury is especially difficult in this area. If in@ury to the facial nerve is not recogni)ed during the operation and the patient awakens with facial paralysis! the surgeon should wait several hours to make sure that this is not a consequence of the local anesthetic. If facial paralysis persists #.2 hours beyond recovery from anesthesia! the packing should be removed. If the surgeon is not absolutely sure that the facial nerve is anatomically intact! the patient should be taken back to surgery immediately! the nerve decompressed around the area of in@ury! and the severity of the in@ury assessed. 7ased on the assessment! the surgeon must decide whether or not to e4cise the in@ured segment. A consultation should be obtained if possible! and consultants should document their assessment and opinion. If the facial nerve was clearly visuali)ed at the time of surgery and the operating surgeon is absolutely certain

that the nerve is anatomically intact! treat the in@ury like any other posttraumatic nerve paralysis. 7egin electroneuronography testing appro4imately ?1 hours postoperatively. If! within the first 1.# weeks! the compound action potential on the affected side drops to less than 0;D of the compound action potential on the normal side! the patient should be taken back to the operating room for e4ploration. *epair If more than 6;D of the diameter of a facial nerve is deemed nonviable because it has been cut! crushed! or stretched! then the in@ured segment should be resected and the nerve should be anastomosed or grafted. If a primary anastomosis can be achieved without tension! this is probably the best method of repair. A primary anastomosis with small segments of missing nerve is possible! but only if the nerve can be mobili)ed and rerouted over a substantial portion of its length. &his is not usually possible when surgery is limited to the mastoid and tympanic segment. If a primary anastomosis without tension cannot be achieved! then an interposition graft should be placed. -onitoring Controversy e4ists as to whether or not facial nerve monitoring is helpful in reducing the risk of postoperative facial nerve in@ury. &he routine use of facial nerve monitoring remains controversial. A 0<<; survey showed that most e4perienced otologists do not believe that facial nerve monitoring is obligatory. Indeed! a large percentage of surgeons use such monitoring only in selected cases. &hese include patients requiring revision surgery! patients who have had perioperative facial nerve weakness in the past! or patients in whom imaging studies show facial nerve anomalies. 'n the other hand! some surgeons believe that predicting the cases in which the facial nerve is at risk is impossible and! consequently! believe monitoring should be performed in every case. &hese surgeons see facial nerve monitoring as a precaution! much like electrocardiographic monitoring! that can potentially be useful in any given case. #otal neurosensory hearing loss A 0.1D chance of total neurosensory hearing loss is associated with cholesteatoma removal. A cholesteatoma that has produced a labyrinthine fistula or that lies directly over the footplate is more likely to produce permanent neurosensory loss./1?3 +ra%t %ailure In appro4imately 0;.06D of patients undergoing cholesteatoma removal! the surgical graft fails! and a tympanic membrane perforation occurs that! if untreated! will be permanent. Such perforations! however! frequently can be eliminated with a revision operation. Balance disturbance Eong.term balance disturbance can occur because of labyrinthine or middle ear in@ury but is uncommon! occurring in less than 0D of patients. Chondritis and perichondritis %erichondritis or chondritis occurs in less than 0D of patients. Care should be taken at the time of surgery to minimi)e the amount of e4posed cartilage because e4posure and devasculari)ation render the cartilage more susceptible to infection.

%erichondritis develops slowly and has the following symptoms$ (0 increasing pain! erythema! and edema of the skin over the involved conchal or auricular cartilage and (1 occasional fluctuance. Hluoroquinolones offer e4cellent coverage for these infections! achieving blood levels equal to those achieved with oral or I9 antibiotic administration. 7ecause the blood supply to cartilage is relatively sparse! however! perichondritis and chondritis are slow to respond to antibiotic therapy. Antibiotic therapy must be continued for a minimum of #.2 weeksA often! = weeks of oral antibiotic therapy is required. If fragments of devitali)ed cartilage become sequestered! they require operative removal. If no clinical response to antibiotic therapy occurs within 1.# weeks or if the infection worsens during that period! operative drainage should be considered. (rains should be left in place for several weeks. A semicircular incision that parallels the margin of the antiheli4 minimi)es the cosmetic impact. Persistent drainage %ersistent drainage from a canal wall:down cavity can arise for a number of reasons. -ost commonly! persistent drainage results from either a sequestered air cell that continues to harbor infection or a small area of osteitis. In such cases! the cavity as a whole heals up quite well e4cept for a small area that remains covered with granulation tissue. &he only solution is to remove the involved area. If the area of osteitis is large and postoperative otorrhea has persisted for months or years! skin grafting should be considered. Skin grafts are especially useful in cavities that have developed mucosal (as opposed to squamous epithelial linings on at least 0 occasion. Sometimes! persistent or recurrent drainage is due to a residual cholesteatoma! in which case the only viable solution is reoperation to remove residual disease. *oreign bodies Horeign bodies retained within the mastoid cavity or wound must be removed if they have become the focus for inflammation or infection. &he most common foreign bodies are small metal fragments that result from the burr hitting the end of the suction irrigation during surgery. *etained pieces or fragments of packing andCor shreds of cotton can similarly serve as a nidus of infection but can usually be removed in the office. "ltered taste -any patients have alteration of taste on the anterior ipsilateral tongue for weeks after an otologic procedure. &his condition! however! usually resolves within a few months after surgery. ,ong)#erm Monitoring An open cavity should be problem.free once it is fully healed! provided that the patient returns every =.01 months for microscopic cleaning in the office. At the cleanings! variable amounts of desquamated epithelium andCor cerumen fill the depths of the cavity. 'ccasional areas of crusting may be present! behind which superficial areas of infection will have developed. +sually! removing the crust and e4posing these areas to circulating air is sufficient to resolve the infection. 5ome care may consist of regular installations of hydrogen pero4ide or a one.to.one mi4ture of alcohol and white

vinegar. Such installations may be administered daily! every other day! or weekly. &he alcohol.vinegar combination is desiccating. 7ecause the skin of the epitheliali)ed cavity is normal squamous epithelium! filling the cavity once or twice a month with an emollient is sometimes helpful. 7aby oil or any other mineral oil product is effective. &his may help to soften cerumen and reduce itching. -ost patients swim comfortably with an open cavity! but some develop vertigo when cold water enters the ear and stimulates the e4posed hori)ontal semicircular canal. In such circumstances! the use of an earplug can effectively the block circulation of water over the e4posed canal and eliminate vertigo. Children should be watched carefully the first several times they swim to make sure that they do not develop dysequilibrium and vertigo while in the water. ,ach patient must be monitored for many years. *ecurrence can occur long after the initial surgical e4cision. Hollow.up care should include semiannual or annual evaluations! even in patients who are asymptomatic. &he frequency with which a patient with a canal wall:down mastoidectomy cavity needs to be seen in order to keep the ear free of desquamated epithelium and cerumen becomes readily apparent. Some patients may require follow.up evaluations as often as every # months for cavity cleaning! while other patients may need to be seen only once a year. %atients who have had canal wall:up operations generally need a second.look procedure =.< months after the original operation. 'nce the second.look operation is healed! regular follow.up care at intervals of = months to 0 year are necessary in order to identify persistence or recurrence of the cholesteatoma. Medication Summary (rug therapy is not currently part of the standard of care for cholesteatomas! being of little use even when the lesions become infected. 7ecause a cholesteatoma has no blood supply! systemic antibiotics cannot reach the center of the lesion. &opical antibiotics can often surround a cholesteatoma! penetrating a few millimeters toward its center and suppressing infection! but a large infected cholesteatoma resists any type of antimicrobial therapy. Consequently! otorrhea either persists or recurs despite frequent aggressive treatment with antibiotics.